Abnormal cytosine–adenine–guanine (CAG) repeats in the ATXN3 gene coding region, resulting in a polyQ expansion containing 60–87 repeats in the mutant ataxin-3 protein, enhances mitochondrial apoptosis, leading to neuronal death in specific brain regions of SCA3 patients [1,2]. This evidence concerns the gene ATXN3 and Spinocerebellar ataxia type 3.